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3e0g

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{{Seed}}
 
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[[Image:3e0g.jpg|left|200px]]
 
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==Structure of the Leukemia Inhibitory Factor Receptor (LIF-R) domains D1-D5==
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The line below this paragraph, containing "STRUCTURE_3e0g", creates the "Structure Box" on the page.
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<StructureSection load='3e0g' size='340' side='right'caption='[[3e0g]], [[Resolution|resolution]] 3.10&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[3e0g]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3E0G OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3E0G FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.1&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr>
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{{STRUCTURE_3e0g| PDB=3e0g | SCENE= }}
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3e0g FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3e0g OCA], [https://pdbe.org/3e0g PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3e0g RCSB], [https://www.ebi.ac.uk/pdbsum/3e0g PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3e0g ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/LIFR_HUMAN LIFR_HUMAN] Defects in LIFR are the cause of Stueve-Wiedemann syndrome (SWS) [MIM:[https://omim.org/entry/601559 601559]; also knowns as Schwartz-Jampel syndrome type 2 (SJS2). SWS is a severe autosomal recessive condition and belongs to the group of the bent-bone dysplasias. SWS is characterized by bowing of the lower limbs, with internal cortical thickening, wide metaphyses with abnormal trabecular pattern, and camptodactyly. Additional features include feeding and swallowing difficulties, as well as respiratory distress and hyperthermic episodes, which cause death in the first months of life. The rare survivors develop progressive scoliosis, spontaneous fractures, bowing of the lower limbs, with prominent joints and dysautonomia symptoms, including temperature instability, absent corneal and patellar reflexes, and smooth tongue.<ref>PMID:14740318</ref> Note=A chromosomal aberration involving LIFR is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(5;8)(p13;q12) with PLAG1.
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== Function ==
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[https://www.uniprot.org/uniprot/LIFR_HUMAN LIFR_HUMAN] Signal-transducing molecule. May have a common pathway with IL6ST. The soluble form inhibits the biological activity of LIF by blocking its binding to receptors on target cells.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/e0/3e0g_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3e0g ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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gp130 is a shared receptor for at least nine cytokines and can signal either as a homodimer or as a heterodimer with Leukemia Inhibitory Factor Receptor (LIF-R). Here, we biophysically and structurally characterize the full-length, transmembrane form of a quaternary cytokine receptor complex consisting of gp130, LIF-R, the cytokine Ciliary Neurotrophic Factor (CNTF), and its alpha receptor (CNTF-Ralpha). Thermodynamic analysis indicates that, unlike the cooperative assembly of the symmetric gp130/Interleukin-6/IL-6Ralpha hexameric complex, CNTF/CNTF-Ralpha heterodimerizes gp130 and LIF-R via noncooperative energetics to form an asymmetric 1:1:1:1 complex. Single particle electron microscopic analysis of the full-length gp130/LIF-R/CNTF-Ralpha/CNTF quaternary complex elucidates an asymmetric structural arrangement, in which the receptor extracellular and transmembrane segments join as a continuous, rigid unit, poised to sensitively transduce ligand engagement to the membrane-proximal intracellular signaling regions. These studies also enumerate the organizing principles for assembly of the "tall" class of gp130 family cytokine receptor complexes including LIF, IL-27, IL-12, and others.
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===Structure of the Leukemia Inhibitory Factor Receptor (LIF-R) domains D1-D5===
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Structural organization of a full-length gp130/LIF-R cytokine receptor transmembrane complex.,Skiniotis G, Lupardus PJ, Martick M, Walz T, Garcia KC Mol Cell. 2008 Sep 5;31(5):737-48. PMID:18775332<ref>PMID:18775332</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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The line below this paragraph, {{ABSTRACT_PUBMED_18775332}}, adds the Publication Abstract to the page
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<div class="pdbe-citations 3e0g" style="background-color:#fffaf0;"></div>
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(as it appears on PubMed at http://www.pubmed.gov), where 18775332 is the PubMed ID number.
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== References ==
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<references/>
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{{ABSTRACT_PUBMED_18775332}}
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__TOC__
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</StructureSection>
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==Disease==
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Known disease associated with this structure: Stuve-Wiedemann syndrome/Schwartz-Jampel type 2 syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=151443 151443]]
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==About this Structure==
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3E0G is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3E0G OCA].
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==Reference==
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Structural organization of a full-length gp130/LIF-R cytokine receptor transmembrane complex., Skiniotis G, Lupardus PJ, Martick M, Walz T, Garcia KC, Mol Cell. 2008 Sep 5;31(5):737-48. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/18775332 18775332]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Garcia, K C.]]
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[[Category: Garcia KC]]
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[[Category: Lupardus, P J.]]
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[[Category: Lupardus PJ]]
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[[Category: Alternative splicing]]
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[[Category: Cell membrane]]
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[[Category: Chromosomal rearrangement]]
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[[Category: Disease mutation]]
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[[Category: Glycoprotein]]
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[[Category: Ig domain]]
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[[Category: Membrane]]
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[[Category: Polymorphism]]
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[[Category: Receptor]]
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[[Category: Secreted]]
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[[Category: Signaling protein/cytokine complex]]
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[[Category: Transmembrane]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Sep 24 10:44:51 2008''
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Current revision

Structure of the Leukemia Inhibitory Factor Receptor (LIF-R) domains D1-D5

PDB ID 3e0g

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